Tethered Cord Syndrome Research Paper

Tethered cord syndrome
Tethered cord syndrome is a stretch-induced functional disorder associated with the fixation (tethering) effect of inelastic tissue on the caudal spinal cord, limiting its movement. This abnormal attachment is associated with progressive stretching and increased tension of the spinal cord as a child ages, potentially resulting in a variety of neurological and other symptoms. Due to the variation of the growth rate of the spinal cord and the spinal column, the progression of neurological signs and symptoms is highly variable. Some individuals present with tethered cord syndrome at birth (so-called congenital), while others develop the symptomatology in infancy or early childhood. Other individuals may not develop any noticeable symptoms until adulthood.
The majority of these cases are mostly developmental, corresponding to the progressive development of excess fibrous connective tissue (fibrosis) in the filum terminale. The filum terminale is a strand of tissue that bridges the spinal cord tip and the tailbone (sacrum). The inelastic structures in children originated from
defective closure of the neural tube (the precursor of the spinal cord) during embryonic development, eventually forming a condition known as spina bifida. Because of its functional (physiological) nature, tethered cord syndrome can be reversible if surgically treated in its early stage.
Symptoms of Tethered Cord Syndrome
The specific symptoms, severity and progression of tethered cord syndrome vary from one individual to another. In most cases, individuals experience symptoms during childhood. In some cases, symptoms are stabilized in childhood, but become apparent only in adulthood. A high percentage of pediatric cases, with tethered cord syndrome show cutaneous tufts of hair, skin tags, dimples, benign fatty tumors, skin discoloration or hemangiomas. Additional symptoms include lower back pain that worsens with activity and improves with rest (although rarely complained by young children, because of inability to express pain), leg pain or numbness, difficulty walking (gait disturbances), foot and spinal deformities, such as abnormal side-to-side curvature of the spine (scoliosis) or hollow low back (exaggerated lordosis), high-arched feet and hammertoes, and less commonly difference in leg strength. Tethered cord syndrome can also cause difficulties with bladder and bowel control. Affected children may experience involuntary urination or defecation (incontinence) and repeated urinary tract infections. Symptoms in children may be slowly progressive. Adult onset of tethered cord syndrome was considered to be rare for many years, but an increasing number of cases have been reported in recent years. This trend is due to improvement in neurological examinations and in the interpretation of imaging studies. Symptoms common to adult tethered cord syndrome include constant, often severe back and leg pain, which may extend to the rectum and genital area in some cases. Progressive sensory and motor deficits may affect the legs potentially resulting in numbness, weakness or muscle wasting (atrophy) in the affected areas. More than 50 percent of the affected individuals experience bladder and bowel dysfunction, manifested by increased frequency or urgency of urination or constipation. In some cases, a fluid-filled cavity (syrinx) is found, sometimes associated with typical signs and symptoms syringomyelia, such as burning pain in the analgesic (painless on examination) area, decreased motor function and loss of muscle mass, or occasional headaches,
Diagnosing tethered cord syndrome
A diagnosis of tethered cord syndrome is made based upon identification of characteristic signs and symptoms (see the symptom section) that can neurologically locate the lesion to be above the attachment of the anomalies to the spinal cord.
For this purpose, a detailed patient history and a thorough clinical evaluation and detailed MRI studies must be carried out. In children, typical imaging features such as a low lying spinal cord and a thickened filum terminale is confirmed by special imaging techniques such as magnetic resonance imaging (MRI) or computed tomography (CT) scan and ultrasound studies.
An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. In addition, demonstration of spina bifida (bony defect of the lamina) supports a diagnosis of tethered cord
syndrome.
In late teenagers and adults, the displacement of the filum located posterior to the cauda equina (a bundle of nerve roots that originate from the lower spinal cord) is a consistent finding. This important feature is proved by the combination of MRI, endoscopy and surgical findings. During CT scanning (a computer tomography) and MRI special techniques are used to create cross-sectional images of vertebrae and nervous system. In some cases, electromyography (EMG) and nerve conduction studies may be used to assess nerve function. EMG is a test that records electrical activity in skeletal (voluntary) muscles at rest and during muscle contractions. The abnormalities in this examination are only shown in patients with an advanced stage of tethered cord syndrome.
Treatment options
In children, surgery to release “untether” the spinal cord is recommended to prevent or reverse progressive neurological symptoms. The type of surgery varies depending on the mechanical causes, such as an inelastic filum, myelomeningocele, lipomyelomeningocele, and dermal sinus. Accordingly, the surgical prognosis varies depending upon the presenting symptoms and tethering-producing anomalies. It has been said that treatment for adult patients with tethered cord syndrome is controversial However, it is clear that in both pediatric and adult patients who have firm evidence of tethered cord syndrome, prompt surgical intervention results in reversal, or at least stabilization, of symptoms in many cases.